Plazmocelulární leukémie – zapomenutá nemoc
| Title in English | Plasma cell leukemia - The forgotten disease |
|---|---|
| Authors | |
| Year of publication | 2019 |
| Type | Article in Periodical |
| Magazine / Source | Klinická onkologie |
| MU Faculty or unit | |
| Citation | |
| Web | http://dx.doi.org/10.14735/amko201940 |
| Doi | http://dx.doi.org/10.14735/amko201940 |
| Keywords | Cytogenetics; Multiple myeloma; Plasma cell leukemia; Plasma cells; Treatment |
| Description | Plasma cell leukemia (PCL) is a rare dis ease and possibly the most aggressive form of monoclonal gammopathy. It is classified into two forms – primary PCL that occurs without a previously identifiable multiple myeloma stage, and secondary PCL that develops from pre-viously dia gnosed multiple myeloma. These two forms have different cytogenetic and molecu-lar profiles, but both forms have an aggressive clinical course. Combinations of different thera-peutic approaches includ ing autologous stem cell transplantation and currently proteasome inhibitors and immunomodulatory drugs are used to treat PCL. Current dia gnostic criteria, developed in the 1970s, may underestimate PCL prevalence; thus, prospective re-evaluation is be ing considered. Purpose: The aim of this study is to review all available information about PCL with an emphasis on dia gnostics, treatment, and circulat ing plasma cells features. Conclu-sion: Although PCL is rare, it is quite a severe dis ease. Current treatments us ing the latest thera-peutics have prolonged patient survival. However, due to the low incidence of PCL, information about the dis ease is very limited and comes mostly from small retrospective studies. Further studies of PCL are needed, because new information could increase in patient survival and our understand ing of its pathogenesis. |